Craniocerebral disproportion: a topical review and proposal toward a new definition, diagnosis, and treatment protocol

Introduction At some point in their lives, patients previously shunted for hydrocephalus may experience chronic, debilitating headaches, despite the fact that their shunts are functioning properly. Previously published reports have suggested that a subset of these patients may be suffering from an iatrogenic craniocerebral disproportion (CCD) and, therefore, may benefit from procedures that expand the available intracranial space. A unified definition of this disorder, however, is lacking. Here, the authors chart the history (including historical terminology) of CCD, review its incidence, describe its signs, symptoms, and associated radiologic findings, and expound upon its pathophysiology. Next, a concise clinical definition of CCD based on the temporal correlation of headaches with the appearance of plateau waves on intracranial pressure (ICP) monitoring is proposed. The authors conclude with a discussion of the various therapeutic strategies employed previously to treat this disorder and present their individualized treatment strategy based upon the simultaneous utilization of ICP monitors and gradual external cranial vault expansion.